Patient With So-Called Primary Reticulum-Cell Sarcoma Of Brain With 6.5 Years Survival, Treated As Meningeal Leukemia

This paper describes the case history of a 43 year-old patient with so-called primary reticulum cell sarcoma of the brain. The CSF contained cells suggestive of leukaemia which, in view of an assumed myelofibrosis, initially seemed also possible to originate from a haematopoietic focus. The patient complained of intermittent violent headaches, which were controlled by spinal taps to release highly cellularised CSF under high pressure and by intrathecal MTX injection, every 4–6 weeks over a period of 4.5 years. In the terminal stage the patient developed paraplegia which, partly on the basis of neuropathological findings, was ascribed to the large accumulated dose of MTX. The tumour proved to be localised in the fornix; the localisation adjacent to the ventricular system made the intermittent cell eruptions in the CSF possible. On the basis of an erroneous diagnostic assumption, a therapy was instituted which resulted in a survival of 6.5 years, which is exceptionally long for a patient with ‘primary reticulum cell sarcoma’ of the brain.